Life-Threatening Variceal Bleed Caused by Secondary Portal Hypertension Controlled by Emergent Splenopneumopexy

Abstract

Introduction: Variceal bleeding is a common and feared complication of portal hypertension. In cases of recurrent bleeding despite pharmacologic therapy and endoscopic variceal ligation, portal decompressive therapy is often pursued. We present a case of a life-threatening variceal bleed caused by portal hypertension secondary to extensive pre-sinusoidal thrombosis as a result of schistosomiasis. The patient's bleeding was managed with emergent splenopneumopexy, a procedure that created a portopulmonary shunt via anastomosis of the splenic veins and pulmonary veins. Case Description: A 49-year-old male with past medical history of schistosomiasis initially presented to a community hospital with hematemesis and hematochezia. An EGD revealed multiple grade 3 esophageal varices that were banded with resolution of bleeding. Three weeks later, the patient had recurrent hematemesis requiring hospitalization. CT of the abdomen/pelvis revealed occlusion of portal, splenic, and superior mesenteric veins. An EGD was repeated but no targets for band ligation were identified due bleeding and presence of clots. A TIPS procedure was attempted but was unsuccessful due to completely occluded vasculature. The patient experienced ongoing hematemesis and hematochezia with hypotension requiring resuscitation. He was stabilized and transferred to our hospital for higher level of care. Upon arrival, he was intubated for airway protection. A Blakemore tube was placed for balloon tamponade and he was taken to the OR for emergent surgery. The patient underwent splenopneumopexy for creation of a portopulmonary shunt as well as transection of the esophagus with primary esophageal anastomosis. The patient did well post-operatively, remaining hemodynamically stable and did not have any further bleeding. The etiology of his pre-sinusoidal thrombosis was thought to be secondary to schistosomiasis given prior history of infection. A hypercoagulable workup was unremarkable and liver biopsy revealed normal hepatic parenchyma. The patient received empiric treatment for schistosomiasis and was discharged 14 days post operatively. Six months after discharge, the patient had not had any further bleeding. Discussion: Splenopneumopexy was first developed in Japan in the 1960s for treatment of portal hypertension in patients with Budd-Chiari Syndrome. The operation induces a portopulmonary shunt via anastomosis of the superior pole of the spleen and exposed pulmonary veins in the left lower lobe. In our patient's case, extensive pre-sinusoidal thrombosis from schistosomiasis precluded successful TIPS procedure and in the setting of refractory variceal bleeding, the patient's life was ultimately saved by emergency decompressive surgery with splenopneumopexy.Figure 1Figure 2